Many of us are learning about Ramsay Hunt syndrome after pop singer Justin Bieber was diagnosed with the condition this summer. Ramsay Hunt syndrome is a rare neurological disorder that causes paralysis of the facial nerve often accompanied by a painful rash in the ear or mouth. It is caused by the varicella zoster virus, or VZV, which causes chicken pox in children and shingles in adults.
Only about five out of 100,000 Americans experience Ramsay Hunt syndrome each year. The syndrome is especially rare in people who have been vaccinated against shingles and chickenpox.
The disease is named for James Ramsay Hunt, a neurologist who first documented its symptoms. Some medical providers refer to the ear rash as herpes zoster oticus. They call the disease Ramsay Hunt syndrome to refer to ear pain, facial paralysis and a rash on the ear or in the ear canal.
A primary care provider can help you understand risks associated with VZV and set up a vaccination, if needed. Primary care providers at the University of Maryland Medical System offer comprehensive health care through the UMMS network of medical providers.
The Connection Between VZV and Ramsay Hunt Syndrome
VZV is an easily transmissible virus. Children who contract chicken pox often make a full recovery. Afterward, the virus stays dormant in the body as a latent infection. This means the virus is present in the body without causing symptoms.
Reactivation of VZV in adults can cause shingles, as well as Ramsay Hunt syndrome. Experts don’t fully understand why the virus reactivates in the body. A compromised immune system from illness, age or stress may cause the virus to reactivate.
Ramsay Hunt syndrome is not contagious the way VZV is. However, the rash associated with the condition may be contagious for those who haven’t had chickenpox or received the chickenpox vaccine.
Preventing Ramsay Hunt Syndrome
There is no known way to prevent getting Ramsey Hunt syndrome, including vaccines. While there is a vaccine for VZV, which can cause Ramsey hunt syndrome, you can still get Ramsey Hunt syndrome if you have been vaccinated.
Signs and Symptoms of Ramsay Hunt Syndrome
Paralysis of the facial muscles caused by nerve palsy is the most recognizable symptom of Ramsay Hunt syndrome. This mimics the symptoms of another condition called Bell’s palsy, which sometimes results in misdiagnosis. Also, symptoms of Ramsay Hunt syndrome may not occur at the same time. These symptoms may include:
- Facial palsy. A paralysis of the facial nerve causes one side of the face to become visibly impaired. Patients may experience facial weakness.
- Rash. A red, painful rash may appear on the outer or inner ear, the soft palate of the mouth and throat or the scalp. The rash may look like small blisters, and its fluid can spread VZV to others. Medical providers may test this rash for the presence of VZV to confirm a diagnosis.
- Ear or neck pain. This pain may be the first sign of the condition and often continues while other symptoms develop. For some the pain is intense.
- Hearing loss. Damage to the auditory nerve can cause temporary hearing loss. In rare cases, this hearing loss may become permanent.
- Hyperacusis. This condition causes sounds to appear louder, sometimes at painful volumes for those experiencing it.
- Nausea or vomiting. These symptoms can appear on their own or accompany vertigo.
- Tinnitus. Ringing in the ear may occur in some patients.
- Vertigo. Dizziness or a spinning sensation may cause discomfort or nausea.
What to Do if You Have Symptoms
If you have facial paralysis, you should seek medical attention urgently, whether it is through your primary care physician, an urgent care center or an emergency room, to confirm a diagnosis and to start medical therapy right away. It is also important for anyone with facial paralysis to seek a follow up consultation right away with an otolaryngologist and/or a facial plastic surgeon who specializes in treating patients with facial paralysis. The follow up is important because they can also confirm the diagnosis and ensure you get the correct medical therapy, physical therapy and potentially surgical therapy.
Treatment Options
Ramsay Hunt syndrome treatment is more about managing symptoms than curing the disease. A patient’s long-term outcome improves the earlier treatment starts. Even with treatment, some symptoms such as hearing loss or facial palsy can still become permanent. For the most effective results, providers may combine treatment methods.
These methods include:
- Antiviral medications and corticosteroids. Antiviral medications help the body resist viral infections like VZV. Corticosteroids relieve inflammation caused by VZV. The earlier these medication therapies start, the more likely they are to be effective.
- Eye care. Facial paralysis may make it difficult for patients to close one of their eyes completely. Providers may prescribe eye coverings and lubricating eye drops to protect the cornea from dryness.
- Medication management. Medication management for vertigo, nausea and pain may help make patients more comfortable.
Prognosis for Ramsay Hunt Syndrome
For most patients, Ramsay Hunt syndrome often resolves within a few weeks to a few months.
However, some prolonged side effects are common. Recovery from facial palsy in Ramsay Hunt syndrome patients is approximately 70 percent. Almost half of patients still experience contractions of their facial muscles after recovery.
Remember, not every patient experiences recovery in the same way. A primary care provider or a neurologist—along with and ear, nose and throat specialist and facial plastic surgeon—can discuss each patient’s prognosis in detail.
